ALS: A Complex Rehab Challenge

On June 2, 1941, Lou Gehrig died of amyotrophic lateral sclerosis (ALS). He was 37 years old.

Nearly 70 years have passed, but to many people, the New York Yankees’ first baseman still epitomizes what they know of the disease: How it takes young adults in the prime of their lives. How it progresses swiftly toward an inevitable end. And how there is no cure.

But like so many other mobility-related conditions, ALS proves quite complex under closer inspection. Those differences from client to client can result in greater challenges for providers trying to create appropriate seating & mobility systems — and demands both creativity and expertise from everyone on the seating & mobility team.

The Physical Presentation of ALS

The ALS Association (ALSA, alsa.org) defines ALS — commonly called Lou Gehrig’s Disease — as “a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord, and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.”

As those motor neurons lose their ability to send messages to muscle fibers — which is what enables us to move our muscles — the person with ALS may notice a variety of symptoms. ALSA cites muscle weakness, especially in the extremities, and weakness during speaking, swallowing or breathing as typical difficulties.

While ALS has a reputation for progressing at a ferocious pace, its earliest symptoms can be surprisingly subtle.

Says Quantum Rehab Clinical Education Manager Jay Doherty, OTR, ATP, about people with yet-undiagnosed ALS, “They might notice that one hand or one leg is a little weaker than the other, or it could be slurred speech. Some other things (they might notice) are muscle twitching, as well as uncontrollable laughing or crying. Usually, it gets to the point where they’re finding they’re really getting clumsy. And that’s when they start to do a little more investigating into what’s going on.”

ATG Rehab’s John Calcavecchia, ATP, specializes in working with ALS clients in the northeastern United States. He works directly with ALS clinics and sees their clients regularly.

“A lot of folks get diagnosed, and they’ve probably already had it for a year or two,” he says.

Calcavecchia says ALS is “so unpredictable,” noting, “It is a disease where some folks get affected in the upper extremities only, some folks in the lower (extremities) only, some folks both parts. They can just have a tingling in their finger, or a drop foot — where they lose their muscles in their ankle, and their foot just can’t pick up, so their gait is dragging.”

In cases of Bulbar ALS — which involves respiration — Calcavecchia says he sees clients who “have trouble speaking, or they can’t talk anymore, but they have good strength in their legs and their arms. The respiratory (form) is the worst — worse than the mobility or physical side of it, because they’re losing their diaphragm and their ability to breathe.”

A small number of ALS clients have the respiratory version of the disease, says Alisa Brownlee, ATP, the national assistive technology consultant to the national ALSA office. She describes them as “People who can’t walk a few feet before they are so out of breath they can’t talk. They might give you two or three words a minute because they’re so out of breath. But they walk, and their arms are just fine.”

The Faces of ALS

Lou Gehrig, nicknamed The Iron Horse for his durability as a baseball Hall of Famer, was just 36 when he was diagnosed with ALS. Perhaps partly for that reason, ALS is often associated with younger adults.

In actuality, ALSA says the average age at diagnosis is 55, and most newly diagnosed patients are between 40 and 70 — though some patients are in their 20s or 30s. ALSA estimates about 30,000 Americans are living with ALS at any one time, and 60 percent are male, though the gender discrepancy narrows as people grow older.

A small percentage of ALS cases is thought to be genetically linked, but 90 percent of patients have no family history of ALS, the association says. Patients who have no ALS in their families are said to have Sporadic ALS, while those with genetic ALS ties are said to have Familial ALS.

ALSA calls ALS “a very difficult disease to diagnose” because, despite a barrage of possible tests — ranging from electromyography and nerve conduction velocity, to blood and urine studies, x-rays and muscle biopsies — neurologists ultimately come to an ALS diagnosis by discounting other possibilities, such as Parkinson’s.

“The first clinical challenge is the difficulty in diagnosis,” says Lois Brown, MPT, ATP, rehab clinical education specialist for Invacare Corp. “There is no definitive test, and therefore the diagnosis is determined by the process of elimination. This makes the onset and progression difficult to predict and therefore the management of the symptoms and progression difficult.”

Experts talk about an average life expectancy of two to five years after diagnosis, though ALSA notes some people with ALS live significantly longer.

“Respiratory issues are the predictor of where the patient is in the progression of the disease,” Brownlee says. “Forced vital capacity tests (FVC) are used to measure the percentage of oxygen and carbon dioxide in the blood. A person with no respiratory problems will have an FVC of 100 percent or more. As ALS progresses, FVC will fall, which is an indication that the disease is affecting the diaphragm.

“FVCs are used as triggers for medical interventions. For example, when a patient has a 50-percent vital capacity, that will usually prompt an advance-discussion talk. When a client reaches 40 percent, they are eligible for hospice. Knowing the client’s FVC number is crucial for clinicians in order to procure equipment before the client signs on to hospice. Once a client is on hospice, Medicare and most private insurances will not fund a wheelchair.”

Managing Pressure & Providing Mobility

Clients with ALS will need — and can benefit from – a wide range of seating & mobility support offerings. But Ann Eubank, OTR/L, ATP, clinical services manager for Permobil, was quick to point out the need for a comprehensive evaluation of the ALS client, who will surely require customized assistive technology.

“What someone with ALS does not want,” she says, “is to go to a pharmacy and get a ‘sit-and-go’ chair – and then they’ve used up their (DME) benefits.”

A full evaluation is critical because ALS clients need more than just mobility assistance.

Calcavecchia says, “When we first introduce mobility to an ALS client, it’s usually for two main reasons.” First: “(To) decrease the risk of pressure sores; they tend to sit in one spot with limited changes in position, so an intervention that combines power positioning with a quality cushion and back supports is essential.”

The second reason, he says, is to reduce the risk of falls caused by poor balance. “When or if they attempt to stand or walk -- with or without support from family or a walker -- they often fall.” Falling, Calcavecchia explains, is “caused by weak upper extremities to support themselves when using a walker or because the foot and ankle become very weak, causing a drop foot (and) losing the heel-toe strike when trying to walk. To prevent this by utilizing a power wheelchair that can accommodate the seating requirements and immediate electronic control elements is the first step.”

Providers working on seating & mobility for ALS clients must also constantly keep progression of the disease in mind.

“Most providers are going to move right into power mobility because of how quickly ALS progresses,” Doherty says. He mentions the potential benefits of being able to provide a good skin-protection cushion that can provide stability and good envelopment for managing pressure, along with a power positioning system.

“When you’re dealing with someone with ALS, there’s a significant amount of atrophy that goes on to the muscles over the years,” Doherty says. “Because of that atrophy and that wasting away, they become more prone to developing a pressure ulcer. When you’re dealing with pressure management, it’s not just the seat cushion you have to provide. You also have to provide the ability to change their position.”

People with ALS retain sensation, even while they’re losing or have lost the ability to control muscle movement. While seating & mobility clients who retain sensation are often anecdotally assumed to be at less risk for skin breakdown than clients who cannot feel and complain about discomfort or pain, Brown points out, “Any patient who is immobile, meaning they can no longer weight shift, is at risk.”

She adds, “Because of the expectation of progressive loss of strength and decreased postural pelvic and trunk support, and expected atrophy which increases exposure of bony prominences, the recommendation of a pressure and postural support cushion is imperative.”

Power positioning — tilt, recline, elevating legrests and seat elevator — are also crucial, Brown notes. “Of course, the cushion alone without power seating is not enough to provide skin protection or functional positioning. If the seating isn’t right, then access to the input type and driving, and access to other features, can be compromised by sliding out of a stable position.”

Weight loss can also impact a seating system’s fit and function. As ALS progresses, eating and swallowing become difficult, and formerly routine activities such as breathing can require much more effort. “We’ve done testing at our center, and sometimes the effort of just breathing alone might soak up 1,800 calories a day in our patients,” Brownlee says.

In those cases, a system with some seat width adjustability and/or the ability to add positioning accessories to the wheelchair back can be helpful to maintain proper positioning.

The Pain Misperception

Brownlee says pain among ALS clients does occur and needs to be addressed as part of the seating & mobility provision process.

“If you read old literature on ALS, it would always say ‘ALS does not cause pain,’” Brownlee says. “This is a fallacy, and clinicians need to understand that pain management is an important component in managing ALS symptoms.”

In ALS clients, she explains, “Pain often radiates in the shoulder from the joint being frozen; the neck from abnormal positioning; and the butt area from lack of weight shifting, whether that is caused by the patient’s inability to access the tilt/space function because of physical inability, which can be exacerbated because they may be left alone for the day and sit in one position all day, or they don’t understand the importance of weight shifting and simply don’t do it.”

Eubank notes that because human beings are supposed to be moving almost constantly, losing that ability has dire consequences.

“Your body wants to move about every 10 seconds neurologically,” she says. “Comfort really is neurological. It’s painful to be in one position. People with ALS have sensation; they can become in pain just as you and I would if we sat in one spot for a long time. So power tilt, recline, seat elevator, power (legrests) are very important.”

Doherty points out that pain and discomfort in a seating system can also indirectly lead to fatigue.

“To sit for any period of time, they become very uncomfortable,” Doherty says of ALS clients. “Quite often, you’ll find if they’re not comfortable, they’re getting out of their seating system.” Those extra, more frequent transfers can be tiring, he points out.

He adds, “You want them to be able to stay in the chair and tolerate sitting in the chair. So the ability of a power recline system to move their joints to remain comfortable, and to change their position as well as use tilt together with power recline for positioning during the day when they’re fatiguing — to allow gravity to hold them back in the seating system — is extremely important.”

One caveat about tilt and recline: As ALS progresses, clients may find it impossible to tilt or recline as far back as they used to because breathing has become more difficult. Says Brownlee, “It’s often not obvious when our patients are short of breath” — making it all the more important for providers to be aware of potential changes.

Ultimately, understanding that pain is caused by immobility — whether the client has sensation or not — is critical to AT intervention for this population, Brown says. “No seat cushion or any seating surface alone is ever sufficient to prevent discomfort or pressure issues. It’s always the ability for mobility, which is the idea of tilt and recline. You have to constantly readjust your own position for good skin integrity and comfort.”

Other AT Interventions

In addition to these fundamental seating & mobility considerations, ALS clients, their families and their complex rehab teams must also factor in the potential need for ventilators and for power chair electronics that can be programmed to handle increasingly complex functions, including driving controls beyond joysticks.

And while complex rehab providers may not be supplying the actual devices, they need to be familiar with alternative and augmentative communications systems.

“One of the items that I consider important is the integration of the electronics with a communication device or computer,” Brownlee says. “Use of the power wheelchair’s electronics to operate a speech device or computer can make access for the patient so much easier and can impact quality of life.

“If a client does not use the electronics on the wheelchair, they can have multiple switches that caregivers need to set up daily for access to things like emergency call devices, speech devices, regular telephones and environmental controls. Having multiple switches can be a burden on caregivers and confusing to the patient. Integrating all these devices with the electronics on the wheelchair is a relatively simple task, but many clinicians are not aware of the wheelchair’s ability to be integrated with external devices. Some clinicians feel that access to a speech device is the specific purview of an SLP (Speech-Language Pathology specialist), but often these professionals do not know the electronics of the wheelchair, so integration never occurs.”

Calcavecchia says ATG doesn’t sell communications devices, “but we work with other suppliers that offer the devices. I can call the communications person and say, ‘What are you using with this client? I want to get the mount that matches that.’”

He adds that he gives the client a comprehensive review of a power chair’s potential.

“When I’m doing an eval with someone who needs a wheelchair today, I am talking about all the power features that come with a chair,” he says. “If they’re appropriate for a power chair, I’m breaking down the tilt, the recline, the power legs, even the seat elevator. I’m discussing all four power features with them.”

He also addresses future needs: “The question always comes up, a hundred out of a hundred times: ‘What if I lose function in my hand, what can I do?’ And then I start going into alternative drives, where the chair can be equipped so you can drive with your head, with your chin, with even a minijoystick, where you can use the tip of your finger to drive the chair. I do try to give them as much information as I can when they ask. I really let them drive the conversation of where they want to go, and I try to give them as much information as possible.”

The issue of ventilation, he admits, “is always more sensitive, depending on how much the family and the patient can hear at one time. I kind of stay away from it unless they ask.”

Because Calcavecchia works directly with specialized ALS clinics, he says the initial explanation of ventilation — how ALS eventually will impact the muscles required for breathing — is done by physicians. “I don’t want to be the one to discuss a vent tray; I want the doctor to bring it up to them,” he says. “Then I come in and say, ‘This is what the doctor spoke about, this is why we need this component on that chair if you want it.’”

The decision of whether or not to use a ventilator when the time comesis often agonizing for patients and their families — and that decision canchange as time goes on. Providers, however, need to face the possibilityof a ventilator while creating the seating & mobility recommendation — which can happen years before.

For that reason, Calcavecchia says he always presumes the system he is creating will need to accommodate a ventilator one day.

“Even if somebody has said they had made a decision — they didn’t want to go on a ventilator — I still have to make sure (the power base) would accept a ventilator,” he says. “Even though they may say no at this point, those chairs can meet a lot of other needs and can allow for a change of mind later on. It’s a major problem if you don’t accommodate for (a ventilator) early on.”

That mindset — building a power mobility system that is as fl exible as possible — seems to be a good idea judging from conversations among ALS patients in blogs and chatrooms, says Eubank. Citing one blog entry written by an ALS client, Eubank notes, “He said, ‘Get everything you can on the chair now; you’ll need it very quickly.’ Even if they can’t drive or they feel uncomfortable driving, they really value even more being able to move their bodies in space. That points to ‘powered everything.’”

A Provider’s Response

The progressive nature of ALS puts suppliers in a tough position of having to both plan for the future and answer needs they see today.

“A seating system is created based on what stage of the disease the patient is in today,” Calcavecchia says. “Power or manual mobility is selected by looking at the strength of both the lower and upper extremities. Unfortunately, not everyone goes directly to power; some who are too far along in the disease progression go into a manual tilt and recline wheelchair.”

He tries to design seating & mobility systems based on three concepts: proper body position/alignment, preparation for future changes and comfort.

“For proper body position, we select components that will support weak extremities,” he says. “For example, we’ll select hip/thigh supports if hip muscles are weak and abduct or fall to the outside. Or often, we’ll choose lateral supports to aid diminishing trunk strength, which will cause the upper body to collapse to one side.”

Next step: Considering future needs. Calcavecchia says, “Future consideration is critical, so the chair selected initially must have the ability to change when a patient’s progression occurs. For example, if power legs are now needed because the client has swelling or is at risk for a DVT (deep vein thrombosis), or if a patient requires mechanical ventilation, the chair needs to be able to support a vent and battery tray to maintain independence.”

A seating & mobility system that meets today’s and tomorrow’s clinical needs must also be comfortable for the client to use. “No matter what the diagnosis, comfort is always a key element in seating & positioning, as the patient needs to be motivated to use the system,” Calcavecchia says.

Of course, delivering the seating & mobility system to the client is typically just the start of the service process for the rehab technology supplier.

“Unfortunately with ALS, the upper and lower extremities are progressively getting worse, and not always at the same time,” Calcavecchia says. “Some patients could have no progression of lower extremities, but their upper extremities are declining rapidly, and vice versa. This progression could require positioning adjustments, electronic access enhancements or major functional adaptations, such as additional power positioning features or mechanical ventilator accommodation.”

When those needs arise, Calcavecchia says, “Our response needs to be immediate, and we foster open communication early in the process so the family feels comfortable keeping us posted on how the equipment is functioning. Upon notification, we need to alert the therapist involved to bring the client back to clinic for another evaluation. New components or power features must be processed, and fortunately at ATG Rehab, we have the ability to expedite these orders with very minimal delay.”

Though ALS clients very often receive power chairs with full arrays of power positioning options, Brown points out that, as with any other complex rehab client, a comprehensive evaluation of the patient’s situation — including living environment and transportation needs is a wise move. For instance, a client using a ramp-style van to commute, she notes, may find the seat elevator adds too much height to clear the van’s door or roof.

While power positioning works well for many ALS clients, for some patients other factors such as being able to get into that van ”may be more important than just building a system because somebody said, ‘Make sure you get everything, make sure they don’t cheat you,’” Brown says. “We’ve developed these sorts of product recommendations that are automatic for ALS, and I think you have to go back to the emotional need, what the family can handle, how much other equipment (is already in the home), where they are in their progression — and really make an individualized recommendation that’s going to work for the patient and the family.”

ALS’ Emotional Toll

While the priority of the seating & mobility professional is to assess for and create an appropriate system for an ALS client’s current and future needs, being able to do so can also require understanding the enormous emotional impact the diagnosis can have.

Brownlee addressed that truth when asked the foremost challenge an ALS client faces: “The ability to accept (the) diagnosis and its impact on their daily lives,” she says. “One of the greatest challenges in using assistive technology (AT) is first learning to accept the need to use a device. Using AT means an individual can no longer do something — walking, talking or accessing a computer — the way they once did.”

This new world of assistive technology, while able to preserve independence for clients, can also be intimidating and require a learning curve. “Use of high-technology AT devices requires the ability to adapt to a new method of doing things, such as replacing a traditional mouse with a mouse used by head movement, or using a power wheelchair instead of a walker or manual wheelchair,” Brownlee points out. “There is, for just about everyone, a reluctance to adopt or advance to a new level of assistive technology until it is absolutely necessary.

“People with ALS frequently wait much too long to get additional support, and that creates big problems. It’s natural to resist using assistive technology because it tends to make a disability visible to the world and also forces a disabled person to look around the corner and consider what they may need in a few weeks or months, which can be overwhelming.”

Calcavecchia says he encounters resistance from clients and caregivers. “This does get difficult when we visit a family’s home with a power wheelchair in tow. The client often feels ‘This is it, I’m confined to wheelchair.’ Their guard goes up and they may close off from the process. I try to create a positive outlook, explaining the chair is used to extend your day so you can be independent without relying on others. Instead of being exhausted by noon, the chair will substantially enhance their independence and interaction with others.”

He also says he tends to get more pushback from older clients and families. “ALS can be for any age — I’ve seen mid-20s up to 80-year-old people. I get more resistance from the 60- or 70-year-old folks. They’re old school, they have their ways: ‘I’ll be fine, I’ll manage it myself.’ Or ‘I’ll take care of my spouse without any device. I can do it. I’ve taken care of her for 30 years, I’ll take care of her now.’”

Calcavecchia says he tries to involve families in the assessment and decision-making process whenever possible, “especially the kids. They are my link to kind of persuade Dad and Mom as to why things are needed. When I do an eval, I ask, ‘Mrs. Jones, will your family be there as well?’ And she’ll say no, and I’ll say, ‘Is there a way that we can get them there?’ I do want those kids involved. So I try to address it as a family type of decision, not just husband and wife or whatever, because it tends to be dominated by the negative until you get the family involved, especially the kids. ‘No, Dad, no, Mom, this is why you need it’ tends to go over a lot more smoothly.”

With younger adults, Calcavecchia says, “They tend to say, ‘Let me get whatever it takes. I just want to be out playing with my kids.’ A lot of folks have young kids, and they say, ‘I just want to be outside.’ They’re much more motivated to get whatever it takes to be active with their kids and the rest of the family. I don’t get too much resistance from them.”

Eubank says that explaining how assistive technology can improve quality of life can provide powerful incentive to clients.

“People (with ALS) are saying (in blogs) that autonomy, when you’re losing things so quickly, is very, very important — (to be able to) sit up and talk to their family members, see a baseball game or whatever it is they want. They want to have value with their family for as long as they can.”

Preserving independence can be a good AT goal as well, she notes:n “Transfers become more difficult over time, so in the bathroom, a person’s going to want to sit on the toilet. No one wants to use a bedpan, plus it’s really difficult to empty your bowels and bladder when you’re just lying down. So it’s always recommended to be in a chair that goes over the toilet or that even rolls into the shower. What’s the most respectful way that that can happen, given the person’s home?”

Getting Additional Support & Assistance

Perhaps the last word on ALS is that it’s too impactful a diagnosis for anyone — client, family member, clinician, provider – to deal with it alone. The more help you can bring in — for yourself and your client – the better.

For example, some wheelchair manufacturers offer expedited processing for equipment quotes and orders if the client has ALS. That prioritizing can help providers to shave weeks off the usual delivery process. ATG Rehab, for instance, works with manufacturers and also has honed its internal processing to be able to expedite deliveries to ALS clients.

Cody Verrett, ATG’s VP of sales & marketing, says the company’s accelerated delivery program comprises “specific documentation for the diagnosis, specific information technology modules that we have within our own systems, supplier relationships that help expedite not only the quote, but also the product order and delivery process. The custom-tailored documentation specific to the diagnosis was created specifically to help expedite this process. We sent up supplier relationships to do the same thing.”

While Eubank says trying to equip an ALS client’s power chair with full power positioning from the very start can be the best move for many clients, she realizes that’s not always possible. In those cases, she says that choosing a power base that enables you to add positioning in a modular fashion can be a real time-saver. “You can add tilt and recline very quickly,” she says. “You do not have to send the chair back to the manufacturer.”

Due to the complex nature of this condition, Brownlee strongly recommends that clinicians and providers seek specialized support: “The thing that I would hope for if they’re dealing with an ALS client is that they would seek out ALS professionals whether or not it’s through an ALS chapter or certified center, because we are the people who can often help patients process all this a little quicker.”

And that support goes both ways, Brown says, explaining that the provider summoned to the ALS client’s home to “fix” a joystick that seems unresponsive is in the best position to alert the rest of the team. “They can let (the clinic) know that the client needs to get back in,” she says, “that they are beyond safely operating that wheelchair or they are beyond using any of the powered seating functions.”

Finally, in addition to seeking the input of members in the client’s health-care team, also consider turning to those who know first-hand what it’s like to live with ALS (see sidebar below).

“The community is vibrant,” Eubank says. “The community offers a lot to each other; they switch and loan equipment, which has its good and bad qualities. But they also offer support to each other.”

Family, specializing clinics, manufacturers who expedite orders, support groups…when the diagnosis is ALS, it’s clear that a team approach is best. “It’s a terrible thing,” Calcavecchia says of ALS. Which is why he says, “I’ll grab any tool I can find on the desk.”

ALS Support Groups: “Things Can Happen Very Quickly”

If you’re looking for more insight into living with ALS, Ann Eubank, OTR/L, ATP, clinical services manager for Permobil — who is a national sponsor of the ALS Association — suggests checking out blogs and chatroom conversations written by people with ALS and their caregivers.

One worth checking out: ALS Chat (alsforums.com), which encourages members to share stories and exchange ideas on topics ranging from caregiving to feeding tube pros and cons (families and patients seemed to agonize over the procedure beforehand, but were quite happy with the benefits afterward).

In one example, a writer with ALS talks about assuring his son that he could be alone for a few minutes while his son stepped outside to take care of a chore. As soon as the son closed the door behind him, a hose on his father’s ventilator detached.

The writer was determined not to put his son through the trauma of coming back inside to find his father dead: “I have never fought so hard for anything in my life.… If it had been anyone but him I would be dead.”

Noting “how quickly things can happen,” the writer said he and his family would be creating a call system to prevent future incidents. Site visitors who commented on the blog expressed relief that the writer was OK, and offered advice such as using an infant monitor as a communicator.

“Please take care,” added one commenter. “We can’t afford to lose you yet.”

This article originally appeared in the May 2010 issue of Mobility Management.